What are the diagnostic criteria for idiopathic pulmonary fibrosis (IPF)?

Updated: Aug 06, 2018
  • Author: Ali Nawaz Khan, MBBS, FRCS, FRCP, FRCR; Chief Editor: Eugene C Lin, MD  more...
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Answer

Answer

According to the American Thoracic Society (ATS), European Respiratory Society (ERS), Japanese Respiratory Society (JRS), and Latin American Thoracic Association (ALAT) evidence-based guidelines for diagnosis and management of IPF,  the criteria for diagnosis are as follows [1]

  • All known causes of interstitial lung disease (ILD) are excluded, including other idiopathic interstitial pneumonias and ILD associated with environmental exposure, medication, or systemic disease.
  • A UIP pattern is present on high-resolution computed tomography (HRCT) in patients not subjected to surgical lung biopsy.
  • Specific combinations of HRCT and surgical lung biopsy pattern in patients subjected to surgical lung biopsy. 

Radiologic characteristics of pulmonary fibrosis appear in the image below.

HRCT of advanced stage of pulmonary fibrosis demon HRCT of advanced stage of pulmonary fibrosis demonstrating reticular opacities with honeycombing, with predominant subpleural distribution.

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